Abstract

Studies of gonadotrophin pulsatility and pelvic ultrasound morphology distinguish between isolated premature thelarche (IPT) and central precocious puberty (CPP) (Eur J Pediatr 1986; 145:190) We have observed 6 girls (age range, 2.2 - 6.9 yrs) with premature sexual development which fitted neither diagnostic category. Features characteristic of IPT were absent pubic hair (n=6), fluctuating breast size (n=4), a small uterus on ultrasound without an endometrial echo (n=6). Features more consistant with CPP were accelerated growth (n=5) and progressive breast enlargement (n=2). Ovarian ultrasound morphology showed that all 6 girls had ovaries containing large numbers of small cysts between 3 - 4 mm in diameter. 5 girls had predominant FSH response to i/v GnRH. The results of overnight serum gonadotrophin secretion were not typical of CPP or IPT. Discrete LH and FSH pulses were seen with neither prcdominating. 5 girls were treated unsuccessfully with intranasal (D-Ser6) GnRH but 2 girls responded to subcutaneous administration. 2 girls had spontaneous regression of breast development after intranasal therapy ceased. We believe that our patients represent a spectrum between IPT and CPP which is relevant for prognosis and treatment as well as for understanding disorders of ovarian maturation.

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